Epidemiology and prognostic factors for mycosis fungoides and Sézary syndrome in a multi‐ethnic Asian cohort: a 12‐year review

摘要

Abstract Background Limited information exists regarding survival of Asian patients with mycosis fungoides ( MF ) and Sézary syndrome ( SS ). Objective To evaluate the epidemiology, outcome and prognostic factors of these patients. Methods A retrospective review of MF / SS cases diagnosed from 2000 to 2011 at a tertiary referral dermatology centre in Singapore was performed. Results Of 246 patients, 63% were male and the median age at diagnosis was 49?years. 73.2% were Chinese, 12.6% Indian, 6.9% Malay and 7.3% Caucasian. A total of 239 patients (97.2%) had MF and seven had SS . Median follow‐up duration was 6.3?years, and median duration of symptoms at diagnosis was 13?months. For patients with MF , the majority had early disease (92.8% stage IA – IIA ). 3.8% were stage IIB , 1.7% stage III and 1.7% stage IV . Complete response to treatment occurred in 78.2%, partial response in 9.6%, persistent but non‐progressive disease in 10.0% and disease progression in 4.1% of patients. Large cell transformation occurred in 4.1% of patients. Mean overall survival during this study was 12.7?years, with death occurring in 2.5% of patients (all ≥stage IIB at diagnosis). For patients with SS , 71.4% presented with stage IVA disease, 28.6% stage IVB . Complete response to treatment occurred in 14.2%, persistent but non‐progressive disease in 28.6% and disease progression in 57.2% of patients. Mean overall survival was 3.3?years within this study, with death occurring in 42.9% of SS patients. Prognostic factors associated with favourable recurrence‐free survival were male gender ( P ?=?0.008), early disease stage (T1) at diagnosis ( P ??0.001) and absence of maintenance treatment after remission ( P ?=?0.01). Conclusion Compared to Caucasian and East Asian cohorts, MF in South‐East Asians was diagnosed at a younger age and associated with lower mortality, largely due to greater prevalence of hypopigmented MF .